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P31040

- SDHA_HUMAN

UniProt

P31040 - SDHA_HUMAN

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Protein

Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial

Gene

SDHA

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli

Functioni

Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor.1 Publication

Catalytic activityi

Succinate + a quinone = fumarate + a quinol.

Cofactori

FAD.

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei275 – 2751FADBy similarity
Binding sitei296 – 2961SubstrateBy similarity
Binding sitei308 – 3081SubstrateBy similarity
Active sitei340 – 3401Proton acceptorBy similarity
Binding sitei407 – 4071SubstrateBy similarity
Binding sitei440 – 4401FADBy similarity
Binding sitei451 – 4511SubstrateBy similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi68 – 736FADBy similarity
Nucleotide bindingi91 – 10616FADBy similarityAdd
BLAST
Nucleotide bindingi456 – 4572FADBy similarity

GO - Molecular functioni

  1. flavin adenine dinucleotide binding Source: InterPro
  2. succinate dehydrogenase (ubiquinone) activity Source: UniProtKB-EC

GO - Biological processi

  1. cellular metabolic process Source: Reactome
  2. nervous system development Source: UniProtKB
  3. oxidation-reduction process Source: UniProtKB
  4. respiratory electron transport chain Source: UniProtKB
  5. small molecule metabolic process Source: Reactome
  6. succinate metabolic process Source: UniProtKB
  7. tricarboxylic acid cycle Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Electron transport, Transport, Tricarboxylic acid cycle

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000073578-MONOMER.
ReactomeiREACT_1785. Citric acid cycle (TCA cycle).
REACT_22393. Respiratory electron transport.
UniPathwayiUPA00223; UER01006.

Names & Taxonomyi

Protein namesi
Recommended name:
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (EC:1.3.5.1)
Alternative name(s):
Flavoprotein subunit of complex II
Short name:
Fp
Gene namesi
Name:SDHA
Synonyms:SDH2, SDHF
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 5

Organism-specific databases

HGNCiHGNC:10680. SDHA.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: UniProtKB
  2. mitochondrial respiratory chain complex II Source: UniProtKB
  3. mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex II deficiency (MT-C2D) [MIM:252011]: A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome.
Note: The disease is caused by mutations affecting the gene represented in this entry.
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti555 – 5551G → E in MT-C2D and CMD1GG. 2 Publications
VAR_016879
Leigh syndrome (LS) [MIM:256000]: An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.2 Publications
Note: The disease is caused by mutations affecting the gene represented in this entry.
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti524 – 5241A → V in LS. 1 Publication
VAR_016878
Natural varianti554 – 5541R → W in LS. 1 Publication
VAR_002449
Cardiomyopathy, dilated 1GG (CMD1GG) [MIM:613642]: A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.1 Publication
Note: The disease is caused by mutations affecting the gene represented in this entry.
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti555 – 5551G → E in MT-C2D and CMD1GG. 2 Publications
VAR_016879
Paragangliomas 5 (PGL5) [MIM:614165]: A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.1 Publication
Note: The disease is caused by mutations affecting the gene represented in this entry.
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti589 – 5891R → W in PGL5; loss of activity. 1 Publication
VAR_065975

Keywords - Diseasei

Cardiomyopathy, Disease mutation, Leigh syndrome, Tumor suppressor

Organism-specific databases

MIMi252011. phenotype.
256000. phenotype.
613642. phenotype.
614165. phenotype.
Orphaneti154. Familial isolated dilated cardiomyopathy.
44890. Gastrointestinal stromal tumor.
29072. Hereditary pheochromocytoma-paraganglioma.
3208. Isolated succinate-CoQ reductase deficiency.
255241. Leigh syndrome with leukodystrophy.
PharmGKBiPA35605.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 4343MitochondrionBy similarityAdd
BLAST
Chaini44 – 664621Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrialPRO_0000010335Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei99 – 991Tele-8alpha-FAD histidineBy similarity
Modified residuei167 – 1671N6-acetyllysineBy similarity
Modified residuei179 – 1791N6-acetyllysine; alternate1 Publication
Modified residuei179 – 1791N6-succinyllysine; alternateBy similarity
Modified residuei182 – 1821N6-acetyllysineBy similarity
Modified residuei215 – 2151Phosphotyrosine; by SRC1 Publication
Modified residuei250 – 2501N6-acetyllysine; alternateBy similarity
Modified residuei250 – 2501N6-succinyllysine; alternateBy similarity
Modified residuei335 – 3351N6-acetyllysine; alternate1 Publication
Modified residuei335 – 3351N6-succinyllysine; alternateBy similarity
Modified residuei480 – 4801N6-acetyllysineBy similarity
Modified residuei485 – 4851N6-acetyllysine; alternateBy similarity
Modified residuei485 – 4851N6-succinyllysine; alternateBy similarity
Modified residuei498 – 4981N6-acetyllysine; alternateBy similarity
Modified residuei498 – 4981N6-succinyllysine; alternateBy similarity
Modified residuei517 – 5171N6-acetyllysineBy similarity
Modified residuei538 – 5381N6-acetyllysine; alternateBy similarity
Modified residuei538 – 5381N6-succinyllysine; alternateBy similarity
Modified residuei541 – 5411N6-acetyllysine1 Publication
Modified residuei547 – 5471N6-acetyllysine; alternateBy similarity
Modified residuei547 – 5471N6-succinyllysine; alternateBy similarity
Modified residuei550 – 5501N6-acetyllysineBy similarity
Modified residuei598 – 5981N6-acetyllysineBy similarity
Modified residuei608 – 6081N6-acetyllysine1 Publication
Modified residuei615 – 6151N6-succinyllysineBy similarity
Modified residuei624 – 6241N6-acetyllysineBy similarity
Modified residuei636 – 6361N6-acetyllysineBy similarity
Modified residuei647 – 6471N6-acetyllysineBy similarity

Post-translational modificationi

Phosphorylation at Tyr-215 is important for efficient electron transfer in complex II and the prevention of ROS generation.1 Publication
Deacetylated by SIRT3.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiP31040.
PaxDbiP31040.
PeptideAtlasiP31040.
PRIDEiP31040.

2D gel databases

REPRODUCTION-2DPAGEIPI00305166.

PTM databases

PhosphoSiteiP31040.

Expressioni

Gene expression databases

BgeeiP31040.
CleanExiHS_SDHA.
ExpressionAtlasiP31040. baseline.
GenevestigatoriP31040.

Organism-specific databases

HPAiCAB034929.
HPA041981.

Interactioni

Subunit structurei

Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD. Interacts with SDHAF2/SDH5; interaction is required for FAD attachment. Interacts with TRAP1.2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
SDHBP219122EBI-1057265,EBI-1056481

Protein-protein interaction databases

BioGridi112290. 67 interactions.
IntActiP31040. 16 interactions.
MINTiMINT-3012212.
STRINGi9606.ENSP00000264932.

Structurei

3D structure databases

ProteinModelPortaliP31040.
SMRiP31040. Positions 52-664.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG1053.
GeneTreeiENSGT00390000010046.
HOVERGENiHBG001461.
InParanoidiP31040.
KOiK00234.
OMAiRDGPILQ.
OrthoDBiEOG7MH0XJ.
PhylomeDBiP31040.
TreeFamiTF300763.

Family and domain databases

Gene3Di1.20.58.100. 1 hit.
3.90.700.10. 1 hit.
InterProiIPR003953. FAD_bind_dom.
IPR003952. FRD_SDH_FAD_BS.
IPR015939. Fum_Rdtase/Succ_DH_flav-like_C.
IPR027477. Succ_DH/fumarate_Rdtase_cat.
IPR011281. Succ_DH_flav_su_fwd.
IPR014006. Succ_Dhase_FrdA_Gneg.
[Graphical view]
PfamiPF00890. FAD_binding_2. 1 hit.
PF02910. Succ_DH_flav_C. 1 hit.
[Graphical view]
SUPFAMiSSF46977. SSF46977. 1 hit.
SSF56425. SSF56425. 1 hit.
TIGRFAMsiTIGR01816. sdhA_forward. 1 hit.
TIGR01812. sdhA_frdA_Gneg. 1 hit.
PROSITEiPS00504. FRD_SDH_FAD_BINDING. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. Align

Isoform 1 (identifier: P31040-1) [UniParc]FASTAAdd to Basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MSGVRGLSRL LSARRLALAK AWPTVLQTGT RGFHFTVDGN KRASAKVSDS
60 70 80 90 100
ISAQYPVVDH EFDAVVVGAG GAGLRAAFGL SEAGFNTACV TKLFPTRSHT
110 120 130 140 150
VAAQGGINAA LGNMEEDNWR WHFYDTVKGS DWLGDQDAIH YMTEQAPAAV
160 170 180 190 200
VELENYGMPF SRTEDGKIYQ RAFGGQSLKF GKGGQAHRCC CVADRTGHSL
210 220 230 240 250
LHTLYGRSLR YDTSYFVEYF ALDLLMENGE CRGVIALCIE DGSIHRIRAK
260 270 280 290 300
NTVVATGGYG RTYFSCTSAH TSTGDGTAMI TRAGLPCQDL EFVQFHPTGI
310 320 330 340 350
YGAGCLITEG CRGEGGILIN SQGERFMERY APVAKDLASR DVVSRSMTLE
360 370 380 390 400
IREGRGCGPE KDHVYLQLHH LPPEQLATRL PGISETAMIF AGVDVTKEPI
410 420 430 440 450
PVLPTVHYNM GGIPTNYKGQ VLRHVNGQDQ IVPGLYACGE AACASVHGAN
460 470 480 490 500
RLGANSLLDL VVFGRACALS IEESCRPGDK VPPIKPNAGE ESVMNLDKLR
510 520 530 540 550
FADGSIRTSE LRLSMQKSMQ NHAAVFRVGS VLQEGCGKIS KLYGDLKHLK
560 570 580 590 600
TFDRGMVWNT DLVETLELQN LMLCALQTIY GAEARKESRG AHAREDYKVR
610 620 630 640 650
IDEYDYSKPI QGQQKKPFEE HWRKHTLSYV DVGTGKVTLE YRPVIDKTLN
660
EADCATVPPA IRSY
Length:664
Mass (Da):72,692
Last modified:November 1, 1995 - v2
Checksum:i180B664E3FFD0B34
GO
Isoform 2 (identifier: P31040-2) [UniParc]FASTAAdd to Basket

The sequence of this isoform differs from the canonical sequence as follows:
     105-152: Missing.

Note: No experimental confirmation available.

Show »
Length:616
Mass (Da):67,300
Checksum:i7F3417D2C871A210
GO
Isoform 3 (identifier: P31040-3) [UniParc]FASTAAdd to Basket

The sequence of this isoform differs from the canonical sequence as follows:
     126-270: Missing.

Note: No experimental confirmation available.

Show »
Length:519
Mass (Da):56,650
Checksum:i608A48ACFF596F2C
GO

Sequence cautioni

The sequence CAA37886.1 differs from that shown. Reason: Differs extensively from that shown.
The sequence BAD92228.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti356 – 3561G → D in AAD51006. (PubMed:10746566)Curated
Sequence conflicti398 – 3981E → D in AAD51006. (PubMed:10746566)Curated
Sequence conflicti591 – 5911A → T in AAD51006. (PubMed:10746566)Curated
Sequence conflicti596 – 5961D → G in AAD51006. (PubMed:10746566)Curated
Sequence conflicti600 – 6001R → Q in AAD51006. (PubMed:10746566)Curated
Sequence conflicti618 – 6181F → L in BAG58722. (PubMed:14702039)Curated
Sequence conflicti640 – 6401E → G in AAD51006. (PubMed:10746566)Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti33 – 331F → V.
Corresponds to variant rs1061518 [ dbSNP | Ensembl ].
VAR_049214
Natural varianti38 – 381D → V.1 Publication
Corresponds to variant rs34635677 [ dbSNP | Ensembl ].
VAR_049215
Natural varianti240 – 2401E → Q.
Corresponds to variant rs1041946 [ dbSNP | Ensembl ].
VAR_049216
Natural varianti333 – 3331V → I.
Corresponds to variant rs1062468 [ dbSNP | Ensembl ].
VAR_059307
Natural varianti524 – 5241A → V in LS. 1 Publication
VAR_016878
Natural varianti554 – 5541R → W in LS. 1 Publication
VAR_002449
Natural varianti555 – 5551G → E in MT-C2D and CMD1GG. 2 Publications
VAR_016879
Natural varianti589 – 5891R → W in PGL5; loss of activity. 1 Publication
VAR_065975
Natural varianti629 – 6291Y → F.3 Publications
Corresponds to variant rs6960 [ dbSNP | Ensembl ].
VAR_071037
Natural varianti657 – 6571V → I.1 Publication
Corresponds to variant rs6962 [ dbSNP | Ensembl ].
VAR_049217

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei105 – 15248Missing in isoform 2. 1 PublicationVSP_055077Add
BLAST
Alternative sequencei126 – 270145Missing in isoform 3. 1 PublicationVSP_055078Add
BLAST

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
D30648 mRNA. Translation: BAA06332.1.
L21936 mRNA. Translation: AAA20683.1.
AF171030
, AF171017, AF171018, AF171019, AF171020, AF171021, AF171022, AF171023, AF171024, AF171025, AF171026, AF171027, AF171028, AF171029 Genomic DNA. Translation: AAD51006.1.
AK291311 mRNA. Translation: BAF84000.1.
AK295937 mRNA. Translation: BAG58722.1.
AB208991 mRNA. Translation: BAD92228.1. Different initiation.
AC021087 Genomic DNA. No translation available.
CH471235 Genomic DNA. Translation: EAW50983.1.
BC001380 mRNA. Translation: AAH01380.1.
BC041016 mRNA. Translation: AAH41016.1.
X53943 mRNA. Translation: CAA37886.1. Sequence problems.
S79641 Genomic DNA. Translation: AAB35332.1.
CCDSiCCDS3853.1. [P31040-1]
PIRiJX0336.
S21302.
RefSeqiNP_001281261.1. NM_001294332.1. [P31040-2]
NP_004159.2. NM_004168.3. [P31040-1]
UniGeneiHs.440475.

Genome annotation databases

EnsembliENST00000264932; ENSP00000264932; ENSG00000073578. [P31040-1]
ENST00000510361; ENSP00000427703; ENSG00000073578. [P31040-2]
GeneIDi6389.
KEGGihsa:6389.
UCSCiuc003jao.4. human. [P31040-1]

Polymorphism databases

DMDMi1169337.

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Web resourcesi

TCA Cycle Gene Mutation Database

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
D30648 mRNA. Translation: BAA06332.1 .
L21936 mRNA. Translation: AAA20683.1 .
AF171030
, AF171017 , AF171018 , AF171019 , AF171020 , AF171021 , AF171022 , AF171023 , AF171024 , AF171025 , AF171026 , AF171027 , AF171028 , AF171029 Genomic DNA. Translation: AAD51006.1 .
AK291311 mRNA. Translation: BAF84000.1 .
AK295937 mRNA. Translation: BAG58722.1 .
AB208991 mRNA. Translation: BAD92228.1 . Different initiation.
AC021087 Genomic DNA. No translation available.
CH471235 Genomic DNA. Translation: EAW50983.1 .
BC001380 mRNA. Translation: AAH01380.1 .
BC041016 mRNA. Translation: AAH41016.1 .
X53943 mRNA. Translation: CAA37886.1 . Sequence problems.
S79641 Genomic DNA. Translation: AAB35332.1 .
CCDSi CCDS3853.1. [P31040-1 ]
PIRi JX0336.
S21302.
RefSeqi NP_001281261.1. NM_001294332.1. [P31040-2 ]
NP_004159.2. NM_004168.3. [P31040-1 ]
UniGenei Hs.440475.

3D structure databases

ProteinModelPortali P31040.
SMRi P31040. Positions 52-664.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 112290. 67 interactions.
IntActi P31040. 16 interactions.
MINTi MINT-3012212.
STRINGi 9606.ENSP00000264932.

Chemistry

ChEMBLi CHEMBL5758.
DrugBanki DB00139. Succinic acid.

PTM databases

PhosphoSitei P31040.

Polymorphism databases

DMDMi 1169337.

2D gel databases

REPRODUCTION-2DPAGE IPI00305166.

Proteomic databases

MaxQBi P31040.
PaxDbi P31040.
PeptideAtlasi P31040.
PRIDEi P31040.

Protocols and materials databases

DNASUi 6389.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000264932 ; ENSP00000264932 ; ENSG00000073578 . [P31040-1 ]
ENST00000510361 ; ENSP00000427703 ; ENSG00000073578 . [P31040-2 ]
GeneIDi 6389.
KEGGi hsa:6389.
UCSCi uc003jao.4. human. [P31040-1 ]

Organism-specific databases

CTDi 6389.
GeneCardsi GC05P000208.
GeneReviewsi SDHA.
H-InvDB HIX0120996.
HGNCi HGNC:10680. SDHA.
HPAi CAB034929.
HPA041981.
MIMi 252011. phenotype.
256000. phenotype.
600857. gene.
613642. phenotype.
614165. phenotype.
neXtProti NX_P31040.
Orphaneti 154. Familial isolated dilated cardiomyopathy.
44890. Gastrointestinal stromal tumor.
29072. Hereditary pheochromocytoma-paraganglioma.
3208. Isolated succinate-CoQ reductase deficiency.
255241. Leigh syndrome with leukodystrophy.
PharmGKBi PA35605.
GenAtlasi Search...

Phylogenomic databases

eggNOGi COG1053.
GeneTreei ENSGT00390000010046.
HOVERGENi HBG001461.
InParanoidi P31040.
KOi K00234.
OMAi RDGPILQ.
OrthoDBi EOG7MH0XJ.
PhylomeDBi P31040.
TreeFami TF300763.

Enzyme and pathway databases

UniPathwayi UPA00223 ; UER01006 .
BioCyci MetaCyc:ENSG00000073578-MONOMER.
Reactomei REACT_1785. Citric acid cycle (TCA cycle).
REACT_22393. Respiratory electron transport.

Miscellaneous databases

GeneWikii SDHA.
GenomeRNAii 6389.
NextBioi 24820.
PROi P31040.
SOURCEi Search...

Gene expression databases

Bgeei P31040.
CleanExi HS_SDHA.
ExpressionAtlasi P31040. baseline.
Genevestigatori P31040.

Family and domain databases

Gene3Di 1.20.58.100. 1 hit.
3.90.700.10. 1 hit.
InterProi IPR003953. FAD_bind_dom.
IPR003952. FRD_SDH_FAD_BS.
IPR015939. Fum_Rdtase/Succ_DH_flav-like_C.
IPR027477. Succ_DH/fumarate_Rdtase_cat.
IPR011281. Succ_DH_flav_su_fwd.
IPR014006. Succ_Dhase_FrdA_Gneg.
[Graphical view ]
Pfami PF00890. FAD_binding_2. 1 hit.
PF02910. Succ_DH_flav_C. 1 hit.
[Graphical view ]
SUPFAMi SSF46977. SSF46977. 1 hit.
SSF56425. SSF56425. 1 hit.
TIGRFAMsi TIGR01816. sdhA_forward. 1 hit.
TIGR01812. sdhA_frdA_Gneg. 1 hit.
PROSITEi PS00504. FRD_SDH_FAD_BINDING. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the flavoprotein (Fp) subunit of liver mitochondria."
    Hirawake H., Wang H., Kuramochi T., Kojima S., Kita K.
    J. Biochem. 116:221-227(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Liver.
  2. "The cDNA sequence of the flavoprotein subunit of human heart succinate dehydrogenase."
    Morris A.A.M., Farnsworth L., Ackrell B.A.C., Turnbull D.M., Birch-MacHin M.A.
    Biochim. Biophys. Acta 1185:125-128(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), VARIANTS PHE-629 AND ILE-657.
    Tissue: Heart.
  3. "Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome."
    Parfait B., Chretien D., Roetig A., Marsac C., Munnich A., Rustin P.
    Hum. Genet. 106:236-243(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT LS VAL-524.
  4. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2), VARIANT VAL-38.
    Tissue: Substantia nigra and Tongue.
  5. "Homo sapiens protein coding cDNA."
    Totoki Y., Toyoda A., Takeda T., Sakaki Y., Tanaka A., Yokoyama S., Ohara O., Nagase T., Kikuno R.F.
    Submitted (MAR-2005) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), VARIANT PHE-629.
    Tissue: Brain.
  6. "The DNA sequence and comparative analysis of human chromosome 5."
    Schmutz J., Martin J., Terry A., Couronne O., Grimwood J., Lowry S., Gordon L.A., Scott D., Xie G., Huang W., Hellsten U., Tran-Gyamfi M., She X., Prabhakar S., Aerts A., Altherr M., Bajorek E., Black S.
    , Branscomb E., Caoile C., Challacombe J.F., Chan Y.M., Denys M., Detter J.C., Escobar J., Flowers D., Fotopulos D., Glavina T., Gomez M., Gonzales E., Goodstein D., Grigoriev I., Groza M., Hammon N., Hawkins T., Haydu L., Israni S., Jett J., Kadner K., Kimball H., Kobayashi A., Lopez F., Lou Y., Martinez D., Medina C., Morgan J., Nandkeshwar R., Noonan J.P., Pitluck S., Pollard M., Predki P., Priest J., Ramirez L., Retterer J., Rodriguez A., Rogers S., Salamov A., Salazar A., Thayer N., Tice H., Tsai M., Ustaszewska A., Vo N., Wheeler J., Wu K., Yang J., Dickson M., Cheng J.-F., Eichler E.E., Olsen A., Pennacchio L.A., Rokhsar D.S., Richardson P., Lucas S.M., Myers R.M., Rubin E.M.
    Nature 431:268-274(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  7. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  8. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 3), VARIANT PHE-629.
    Tissue: Colon and Placenta.
  9. "Cloning of the flavoprotein subunit of human succinate dehydrogenase."
    Malcovati M., Marchetti L., Zanelli E., Tenchini M.L.
    (In) Curti B., Ronchi S., Zanetti G. (eds.); Flavins and flavoproteins 1990, pp.727-730, Walter de Gruyter, Berlin (1991)
    Cited for: PRELIMINARY PARTIAL NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Placenta.
  10. "Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency."
    Bourgeron T., Rustin P., Chretien D., Birch-MacHin M.A., Bourgeois M., Viegas-Pequignot E., Munnich A., Roetig A.
    Nat. Genet. 11:144-149(1995) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 546-562, VARIANT LS TRP-554.
  11. "Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes."
    Aboulaich N., Vainonen J.P., Stralfors P., Vener A.V.
    Biochem. J. 383:237-248(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 76-92 AND 398-418.
    Tissue: Adipocyte.
  12. Cited for: INTERACTION WITH SDHAF2.
  13. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
    Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
    Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-179; LYS-335; LYS-541 AND LYS-608, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  14. Cited for: FUNCTION, VARIANT PGL5 TRP-589, CHARACTERIZATION OF VARIANT PGL5 TRP-589.
  15. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  16. "Mitochondrial c-Src regulates cell survival through phosphorylation of respiratory chain components."
    Ogura M., Yamaki J., Homma M.K., Homma Y.
    Biochem. J. 447:281-289(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION AT TYR-215.
  17. "The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenase."
    Sciacovelli M., Guzzo G., Morello V., Frezza C., Zheng L., Nannini N., Calabrese F., Laudiero G., Esposito F., Landriscina M., Defilippi P., Bernardi P., Rasola A.
    Cell Metab. 17:988-999(2013) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH TRAP1.
  18. "Homozygous Gly555Glu mutation in the nuclear-encoded 70 kDa flavoprotein gene causes instability of the respiratory chain complex II."
    Van Coster R., Seneca S., Smet J., Van Hecke R., Gerlo E., Devreese B., Van Beeumen J., Leroy J.G., De Meirleir L., Lissens W.
    Am. J. Med. Genet. A 120:13-18(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: VARIANT MT-C2D GLU-555.
  19. "Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase."
    Levitas A., Muhammad E., Harel G., Saada A., Caspi V.C., Manor E., Beck J.C., Sheffield V., Parvari R.
    Eur. J. Hum. Genet. 18:1160-1165(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: VARIANT CMD1GG GLU-555.

Entry informationi

Entry nameiSDHA_HUMAN
AccessioniPrimary (citable) accession number: P31040
Secondary accession number(s): A8K5J6
, B4DJ60, E9PBJ5, Q16395, Q59GW8, Q8IW48, Q9UMY5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 1, 1993
Last sequence update: November 1, 1995
Last modified: October 29, 2014
This is version 171 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3